Ambulatory Duchenne muscular dystrophy children: cross-sectional correlation between function, quantitative muscle ultrasound and MRI.

Autor:	Abdulhady H; Department of Physical Medicine, Rheumatology and Rehabilitation, Faculty of Medicine, Ain Shams University, Cairo, Egypt., Sakr HM; Department of Diagnostic and Interventional Radiology and Molecular Imaging, Faculty of Medicine, Ain Shams University, Cairo, Egypt., Elsayed NS; Department of Medical Genetics, Faculty of Medicine, Ain Shams University, Cairo, Egypt., El-Sobky TA; Division of Pediatric Orthopedics, Department of Orthopedic Surgery, Faculty of Medicine, Ain Shams University, Cairo, Egypt., Fahmy N; Neuromuscular Unit, Department of Neuropsychiatry, Faculty of Medicine, Ain Shams University, Cairo, Egypt., Saadawy AM; Department of Diagnostic and Interventional Radiology and Molecular Imaging, Faculty of Medicine, Ain Shams University, Cairo, Egypt., Elsedfy H; Department of Medical Genetics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Jazyk:	angličtina
Zdroj:	Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology [Acta Myol] 2022 Mar 31; Vol. 41 (1), pp. 1-14. Date of Electronic Publication: 2022 Mar 31 (Print Publication: 2022).
DOI:	10.36185/2532-1900-063
Abstrakt:	Duchenne muscular dystrophy (DMD) is a progressive genetic muscle disease. Quantitative muscle ultrasound (US), muscle MRI, and functional tools are important to delineate characteristics of muscle involvement. We aimed to establish correlations between clinical/functional and above-named imaging tools respecting their diagnostic and prognostic role in DMD children. A cross-sectional retrospective study of 27 steroid-naive, ambulant male children/adolescents with genetically-confirmed DMD (mean age, 8.8 ± 3.3 years). Functional performance was assessed using motor function measure (MFM) which assess standing/transfer (D1), proximal (D2) and distal (D3) motor function, and six-minute walk test (6MWT). Imaging evaluation included quantitative muscle MRI which measured muscle fat content in a specific location of right rectus femoris by mDixon sequence. Quantitative muscle US measured right rectus femoris muscle brightness in standardized US image as an indicator of muscle fat content. We found a highly significant positive correlation between the mean MFM total score and 6MWT (R = 0.537, p = 0.007), and a highly significant negative correlation between fat content by muscle US and MFM total score (R = -0.603, p = 0.006) and its D1 subscore (R =-0.712, p = 0.001), and a significant negative correlation between fat content by US and 6MWT (R = -0.529, p = 0.02), and a significant positive correlation between muscle fat content by mDixon MRI and patient's age (R = 0.617, p = 0.01). Quantitative muscle US correlates significantly with clinical/functional assessment tools as MFM and 6MWT, and augments their role in disease-tracking of DMD. Quantitative muscle US has the potential to act as a substitute to functional assessment tools. Competing Interests: Conflict of interest statement None of the Authors has any conflict of interest to disclose. No benefits in any form were received or will be received from a commercial party related directly or indirectly to the subject of this article. (©2022 Gaetano Conte Academy - Mediterranean Society of Myology.)
Databáze:	MEDLINE
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